Purpose: To report a case of necrotizing scleritis associated with Epstein-Barr virus (EBV) infection. Case report: A 68-year-old woman with a history of scleral ulcer, perforation, and scleral graft, with initial negative studies for infectious processes and autoimmune systemic-diseases consulted our service for a second opinion after 2 years of treatment without improvement. She presented severe conjunctival and scleral hyperemia, vascular tortuosity in 360°, areas of scleral thinning and an elevated lesion of nodular appearance. EBV serologic antibody IgG titers were strongly positive and IgM titers were borderline. A scleral biopsy showed granulation tissue with severe inflammation without the presence of tumor cells; PCR was positive for EBV. The patient was treated with antiviral therapy with resolution of the scleritis. Conclusions: EBV although rare, should be considered as a differential diagnosis in patients with necrotizing scleritis with no improvement to conventional therapy.